DEGENERATIVE DISEASESPenyakit degeneratif SSP dapat mengenai :1. Substansia abu2. Substansia putih3. Kombinasi

Daerah yang terkena :- Sering kali difus- Biasanya satu area atau sistem lebih dari lainnya

KLASIFIKASI ANATOMIS

KLASIFIKASI ANATOMIS

I. Diseases of the gray matterII. Hereditary diseases of white matterIII. Neuroectodermal degeneration – phakomatosesIV. Diseases of mitochondriaV. Disorders of cerebrospinal fluid and circulation

I. DISEASES OF GREY MATTER

DISEASES OF THE CEREBRAL CORTEX :

DEMENTIA

DISEASES OF GRAY MATTERDISEASES OF THE CEREBRAL CORTEX.

DEMENTIA

- A progressive deterioration of intellectual capacity resulting from diseases of the brain- 80% due to Alzheimer disease and multi-infarct dementia- The third leading of death in old age group- Clinical manifestation :

- Memory impairment (predominant) – and impaired of :- Language- Perception- Visuospatial function - Calculation- Judgment - Abstraction - Problem solving skills

DISEASES OF GRAY MATTER ( lanj’ )

DISEASES OF THE CEREBRAL CORTEX.DEMENTIA- In Degenerative Disease, the etiologies are unknown, but dementia is a predominant sign- The group of diseases with dementia :

- Alzheimer disease- Pick disease- Multisystem disease- Progressive supranuclear palsy- Huntington disease- Lewy body disease- Cortical basal degeneration- Creutzfeldt-Jacob disease- Fronto-temporal dementia- Normal Pressure Hydrocephalus (NPH)- Vascular dementia

Evaluation of Patients with Dementia1. The history obtained from the patient and other ones

Early cases : punctuated by denial Advanced cases : sparse, fragmented, hesitant

2. The neurological examination MMSE Primitive reflexes Hypertonia or paratonia Dyspraxia Abnormal in reflexes examination

3. The general physical examination Important to exclude signs of: hypoparathyroidism, vascular disease with hypertension, cardiac murmurs, carotid bruit, Vit B12 deficiency 4. Diagnostic procedures

CAUSES OF TREATABLE DEMENTIATherapeutic drug use :

- Anticholinergics – atropine and related comp[ounds- Anticonvulsants : phenytoin, mephenytoin, barbiturates- Antihypertensives : clonidine, methyldopa, propanolol- Psychotropics : haloperidol, lithium carbonate, phenothiazine- Miscellaneous : disulfiram, bromides, paraldehyde, quinidine

Metabolic – systemic disorders :Elctrolyte or acid-base disorders, hypo/hyperglycemia, severeanemia, polycythemia vera, hyperlipidemia, hepatic failure,uremia, pulmonary insufficiency, hypopituarism, thyroid, adrenalor parathyroid dysfunction, cardiac dysfunction, hepatolenticulardegeneration

Intracranial disorders :Cerebrovascular insufficiency, chronic meningitis or encephalitis,neurosyphilis, HIV, epilepsy, tumor, abscess, subdural hematomas, multiple sclerosis, NPH

CAUSES OF TREATABLE DEMENTIA ( lanj’ )

Deficiency states :Vitamin B12 deficiency, folate deficiency, pellagra (niacin)

Collagen-vascular disorders :SLE, temporal arteritis, sarcoidosis, Behcet’s syndrome

Exogenous intoxication :Alcohol, CO, organophosphates, toluene, trichloroethylene, carbon disulfide, lead, mercury, arsenic, thallium, manganese, nitrobenzene, anilines, bromine hydrocarbone

EVALUATION OF THE PATIENT WITH DEMENTIAComplete history,neurological and general physical

examination

Special emphasis onhistory of drug use

evidence of intoxication,evidence of

systemic disease

Neuropsychologicalexamination

CBC, sedimentation rate,electrolytes, BUN, creatinine,glucose, calcium, phosphate, liver enzymes, vit.B12, folate, thyroid function, serologic testfor HIV and syphilis, urinalysis

ECG, CT-scan, MRI,MRA, MRI-spectroscopy,

SPEC, PET

EEG, AEP, VEPIf available

LP, radioisotopecisternography

if available

Consider cerebral angiographyif CT-scan equivocal of if CVD or

arteritis is suspected

Metastatic work-upif indicated

If indicated –blood gases,

heavy metal andintoxication screen

Gilroy J, 2000

ALZHEIMER DISEASE

- Most common form of dementia

- Caused by progressive neuronal degeneration

- 70% in middle age and elderly individuals

- 1% in age 50-70 years, 50% in very elderly people

- The etiology is unknown

- USA : prevalence is 4 million people over age 65 years

DISEASES OF GRAY MATTER :

ALZHEIMER DISEASEPATHOLOGICAL APPEARANCE

☺ Amyloid plaquesconsist of β-amyloid peptide (a fragment of precursor protein)

☺ Neurofibrillary tanglesa core of an abnormally phosphorylated form of the microtubule binding protein tau

DSM-IV DIAGNOSTIC CRITERIA FOR ADA. The development of multiple cognitive deficits manifested by both : 1). memory impairment 2).One (or more) of the following cognitive disturbances : a). aphasia, b). apraxia, c). agnosia, d). disturbance in executive functioning ( planning, organization, sequencing, abstracting )B. The cognitive deficits in criteria A1 and A2 each cause severe impairment in

social or occupational functioning and represent a major decline from a previous level of functioning

C. The course is characterized by gradual onset and continuing cognitive decline

D. The cognitive deficits in criteria A1 and A2 are not due to any of the following: 1).other central nrvous system conditions that cause progressive deficits in memory and cognition (for example, cerebrovascular disease, Parkinson’s disease, Huntington’s disease, subdural hematoma, NPH, brain tumor), 2).systemic conditions known to cause dementia (for example: hypothyroidism, vit.B12 and folic acid deficiency, niacin deficiency, hypercalcemia, neurosyphilis, HIV infection)

E. The deficits do not occur exclusively during the course of deliriumF. The disturbance is not better accounted for by any other Axis I disorder (for

examp-le: major depressive disorder, schizophenia)

American Psychiatric Association (APA): Diagnostic and Statistic Manual of Mental Disorders 4 th ed, 1994

TREATMENTS

1. Diet2. Adequate care3. Planning with family4. Pharmacotherapy :

Sedation and antidepressant Drug therapy (Tacrine, Donepezil HCl)